Acute Disseminated Encephalomyelitis: Current Perspectives

Abstract

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated central nervous system (CNS) disorder, characterized by polyfocal symptoms, encephalopathy and typical magnetic resonance imaging (MRI) findings, that especially affects young children. Advances in understanding CNS neuroimmune disorders as well as the association of myelin oligodendrocyte glycoprotein antibody (MOG-Ab) with both monophasic and recurrent forms of ADEM have led to new insights into its definition, management and outcome. In this review, we aim to provide an update based on current epidemiologic, clinical, radiological and immunopathological aspects and clinical outcome of ADEM.

Update on Acute Disseminated Encephalomyelitis in Children and Adolescents

“…These patients are more likely to have complete clinical-radiological resolution after steroid therapy. Medullary involvement in subjects with anti-MOG antibodies appears to be characterized by longitudinal transverse myelitis [ 2 ].…”

“…The involvement of the peripheral nervous system is more frequent in adult patients [19]. Pediatric patients with ADEM and anti-MOG antibodies usually present with encephalopathy and multifocal neurologic symptoms [2]. These patients are more likely to have complete clinical-radiological resolution after steroid therapy.…”

“…The presence of several conditions mimicking ADEM, added to the lack of specific biomarkers, makes diagnosis potentially hard. Prompt diagnosis is necessary to start adequate treatment to improve the clinical course and long-term outcome [ 2 ]. Because of its heterogeneity in both clinical presentation and course, in addition to the absence of outcome predictive factors, the therapeutic approach differs from case-to-case.…”

“…Because of its heterogeneity in both clinical presentation and course, in addition to the absence of outcome predictive factors, the therapeutic approach differs from case-to-case. Currently, case reports, small observational studies, and expert opinions are the only basis for therapy [ 2 ]. Indeed, the low incidence of this disease and its frequent spontaneous recovery make it difficult to conduct large-sized, randomized controlled studies.…”

“…Actually, ADEM treatment is based on nonspecific immunotherapy, in accordance with the supposed pathogenesis of the syndrome itself and the analogy with multiple sclerosis (MS) [ 2 ]. Indeed, although the exact pathogenesis is still not completely known, the most accurate hypothesis is about immune-mediated damage to the CNS.…”